Joubert综合征的MR诊断价值  

Diagnostic Value of MR to Joubert Syn-drome

作　　者：翟少智[1] 刘国荣[1] 蓝博文[1] 肖俊强[1] 

机构地区：[1]广东省惠州市中心人民医院放射科,广东惠州516001

出　　处：《中国CT和MRI杂志》2013年第1期108-110,共3页Chinese Journal of CT and MRI

摘　　要：目的探讨MR对Joubert综合征的诊断价值。材料和方法回顾性分析经临床随访最后证实的7例Joubert综合征患儿临床和MR资料,7例均行MR平扫。结果 7例患儿均可见以下典型MR表现:小脑蚓部发育不全,左右小脑分开,横断位呈"中线裂"征;第四脑室形态异常,7例横断位呈"蝙蝠翼"状,2例可见"三角形"状;小脑上脚平行走行并增厚,矢状位示小脑上脚垂直走行并增粗,横断位呈"磨牙征"。结论 Joubert综合征具有特征性的MR影像表现,MR检查能清楚显示小脑正中"中线裂"征、"蝙蝠翼"征和"三角形"征,以及中脑水平的"磨牙征",结合临床病史,可以对上述典型表现病例作出准确的定性诊断。Objective To explore the diagnosis value of MR to Joubert syndrome. Materials and methods Clinical and MR data of 7 cases with Joubert syndrome which were clinically confirmed were retrospectively analyzed. All 7 cases were underwent MR regular scanning. Results All 7 cases showed typical MR features： vermis hypoplasia： ＂midline cleft＂ be- tween cerebellarhemispheres in cross section; forth ventricle morphologic abnormality. 7 cases showed ＂bat-wing＂ and 2 cases showed ＂triangular-shaped＇＂ in cross section： supe- rior cerebellar peduncle thickening, ＂molar tooth sign＂ in cross section. Conclusion Joubert syndrome has typical MRI features. MR can show ＂＇midline cleft＂, ~bat-wing＂. and ＂＇trian- gular-shaped＇＂ at cerebellar midline, and ＂＇molar tooth sign＂ at mesencephalon level. Com- bined with clinical history, patients can be qualitative diagnosed accurately.

关 键 词：JOUBERT综合征 磁共振成像 儿童 

分 类 号：R742.8[医药卫生—神经病学与精神病学] R445.2[医药卫生—临床医学]